The difficult management of patients with respiratory segmental dystonia

Respiratory dystonia is a rare and difficult to diagnose disorder, that causes breathing restriction of various degrees. The objective of the study is to report the case of a patient with respiratory dystonia involving the larynx and the pharynx and its evolution concerning spasms intensity and control. CASE REPORT: A 24 year-old-man has been followed for 5 years. The diagnosis was made by means of nasofibroscopy and electromyography. Treatment was carried out with laryngeal and pharyngeal Botulin toxin injections, as it became necessary for symptoms control. CONCLUSION: The difficult management can be secondary to the lack of knowledge on the etiology and physiopathology of the impairment, and because of the limitations in the treatment of associated respiratory symptoms.A forma respiratória da distonia laríngea é rara, de difícil diagnóstico e provoca restrição respiratória de graus variados. O objetivo deste trabalho é apresentar um caso de distonia respiratória envolvendo laringe e faringe e sua evolução em relação à intensidade dos espasmos e seu controle. ESTUDO DE CASO: Paciente de 24 anos, sexo masculino, acompanhado por 5 anos: diagnóstico por nasofibroscopia e eletromiografia e tratamento com toxina botulínica conforme necessidade de controle dos sintomas. CONCLUSÃO: O difícil manejo se deve ao fato do desconhecimento da etiologia e a pouca opção de tratamento, bem como do envolvimento da função respiratória.Universidade Federal de São Paulo (UNIFESP) Escola Paulista de MedicinaPontifícia Universidade Católica de São Paulo Escola Paulista de MedicinaEscola Paulista de Medicina Departamento de Oftalmo/OtorrinolaringologiaEscola Paulista de Medicina Departamento de Otorrinolaringologia e Distúrbios da Comunicação HumanaFundação para o Estudo e Tratamento das Deformidades Crânio-FaciaisUNIFESP CEVUniversidade Federal de São Paulo (UNIFESP) EPM Departamento de Otorrinolaringologia e Cirurgia de Cabeça e PescoçoUNIFESP-EPM Departamento de Otorrinolaringologia e Cirurgia de Cabeça e PescoçoUNIFESP, EPMPontifícia Universidade Católica de São Paulo Escola Paulista de MedicinaEscola Paulista de Medicina Depto. de Oftalmo/OtorrinolaringologiaEscola Paulista de Medicina Depto. de Otorrinolaringologia e Distúrbios da Comunicação HumanaUNIFESP, CEVUNIFESP, EPM, Depto. de Otorrinolaringologia e Cirurgia de Cabeça e PescoçoUNIFESP, EPM Depto. de Otorrinolaringologia e Cirurgia de Cabeça e PescoçoSciEL

Hypoglossal nerve trunk stimulation: electromyography findings during drug-induced sleep endoscopy: a case report

Abstract Background Literature has demonstrated hypoglossal nerve stimulation to be a safe and effective treatment for patients with obstructive sleep apnea nonadherent to positive airway pressure therapy. However, the recommended criteria for patient selection are still unable to identify all the unresponsive patients, highlighting the need for improved understanding about hypoglossal nerve stimulation for obstructive sleep apnea. Case presentation A 48-year-old Caucasian male patient with obstructive sleep apnea had been successfully treated with electrical stimulation of the hypoglossal nerve trunk, documented by level 1 polysomnography data. However, due to snoring complaints, he underwent postoperation drug-induced sleep endoscopy for evaluation of electrode activation during upper airway collapse, aiming to improve electrostimulation parameters. Concurrent surface electromyography of the suprahyoid muscles and masseter was obtained. Activation of electrodes 2, 3, and 6 promoted upper airway opening most strongly at the velopharynx and tongue base during drug-induced sleep endoscopy. The same channels also significantly increased the electrical activity on suprahyoid muscles bilaterally, but predominantly on the stimulated side (right). The masseters also presented a considerable asymmetry in electrical potential on the right side (> 55%). Conclusion Beyond the genioglossus muscle, our findings demonstrate recruitment of other muscles during hypoglossal nerve stimulation, which may be attributed to the electrical stimulation of the nerve trunk. This data provides new insights on how stimulation of the hypoglossal nerve trunk may contribute to obstructive sleep apnea treatment